Cleft Lip

Dr.Mgs. Roni Saleh,

I. Embryology
A.

B.

C.

The critical developmental period of the lip and primary palate occurs during weeks 4 to 6 of gestation. Failure of complete union of the medial nasal prominence and the maxillary prominence leads to a variable extent of clefting of the primary palate, invoiving the upper lip, alveolus, and anterior hard palate to the incisive foramen. Cleft lip alone (CL) and cleft lip and palate (CLP) are considered to be the same entity along a morphologic continuum. Clef palate alone (CF), on the other hand, has different demographics.

II. Epidemiology and Genetics

A.

Incidence of cleft lip and of cleft lip and palate

1.
2. 3. 4.

The overall incidence is 1 in 1,000 live births White ancestry: 1 in 750 live births Asian ancestry: 1 in 500 live births. African ancestry: 1 in 2,000 lilve births.
Male-to-female ratio of 2:! The ratio of left (L) to right to bilateral (B) clefts (L:R:B): 6:3:1 The ratio of CLPmto CL is 2:1 Three percent are syndromic.

B.

Demographics
1. 2. 3. 4.

II. Epidemiology and Genetics (Contd)

5.

Risk factors
a. b. c. d.

Medications: Phenytoin, methylprednisolone (Solo-Medrol), steroids, phenobarbital, diazepam, and isotretinoin. Smoking Parental age, especially fathers age, or both mother and father over 30 years old. Family history (see Genetics)

C.

Genetics
1.
a. b. c. d.

The risk of having a child with CLP

If If If If

parents have one child with CLP: 4% one parent has CLP: 2% to 4% parents have two children with CLP:9% one child and one parent have CLP: 14% to 17%.

2.

Most cases are sporadic(and multifactorial), but may be X-linked, autosomal dominant (Van der Woudes syndrome) of familial (see Syndromes Associatied with Cleft Lip and Palate, later in this chapter).

III. Anatomy
A.

Normal lip anatomy

1.

Topographic landmarks
a. b. c. d. e. f.

g.
h.

Nasal alae. Columella Philtral columns. White roll: Well-defined mucocutaneus or vermilioncutaneous border. Vermilion: Red portion of lip. Tuberle. Cupids bow Wet-dry border: The vermilion-mucosa junction is the border between keratinized and nonkeratinized mucosa.

III. Anatomy (contd)

2.

Musculature
a.

Orbicularis oris
Fibers cross (decussate) in the midline and create the opposite philtral columns. 2) Functions as a sphincters (deep fibers) and for speech (superficial fibers).
1)

b.

Levator labli superioris.

Inserts into the dermis at the vermilion vorder and the lower edge of the philtral columns. 2) Elevates the upper lip.
1)

c.

Nasalis or depressor septi nasi muscle: The fibers run from the alveolar bone into the medial crural footplates, skin of the columelia and the tip of the nose, and into the opposite philtral columns.

III. Anatomy (contd)

3.

Normal measurements.
a.

Vertical length (height) of the upper lip

Newborn: 10 mm. 2) Age 3 months: 13 mm. 3) Adult: 17 mm
1)

b.

The distance between the peaks of Cupids bow: Aprroximately 3 mm at 3 months

4. 5. 6.

Arterial blood supply: The labial artery, bilaterally. Sensory Innervation: the trigeminal nerve, cranial nerve (CN) V, maxillary division (V2). Motor Innervation: the facial nerve, CN VII, zygomatic and buccal branches.

III. Anatomy (contd)

B.

Cleft llip anatomy

1.

Alterations in the orbicularis oris, levator labii, and nasalis result in disruption of continuity, orientation, and quality of the muscless.
Fibers are disoriented and run parallel to the cleft margin. Fibers insert into the alar base on the cleft (lateral) segment and into the columella in the non cleft (medial)segment, as well as intradermally. Incomplete clefts.
1)

a. b.

c.

Simonarts band consist of a skin bridge across the nasal sill. It does not usually contain any significant muscle mass. 2) Some fibers may cross the cleft, if the cleft is less than twothirds of lip height.
d.

Bilateral complete clefts: No muscle tissue is present in the pro-labium.

III. Anatomy (contd)

Vertical lip length is decreased: Cupids bow and the lip are rotated cephalad on both the lateral, cleft side as well as the medial side. 3. Disrupted Cupids bow. 4. The alveolus and nostril floor are open in a complete cleft lip. 5. The premaxilla is rotated and protruding, especially in bilateral cleft lip, often with collapase of the lateral segment of the cleft side(s).
2.

III. Anatomy (contd)

6.

Associated cleft lip nasal abnormalities

a.
b. c. d. e. f. g.

Hypoplastic, flattened alar dome on the affected side. Lack of upper lateral cartilage overlap of lower lateral cartilage. Subluxed lower lateral cartilage with alar base displaced cephalad and posteriorly. Hypoplastic bony foundation (maxilla). The caudal septum is pulled toward the noncleft side. Flattening of the nasal bones. Shortened columella, especially in bilateral cases.

IV. Classification
A.

Extent of the cleft: Complete versus incomplete

1.

Complete cleft lip

a. b.

Complete disruption of the soft tissues to the nasar floor. Tends to be wider than incomplete celfts, with greater nasal deformities. Disruption of the soft tissues to varying degrees. The alveolus is usually intact, with less of a tendency for the premaxilla to protrude. Forme fruste: A very mild cleft.
May be difficult to detect 2) May appear as vermilion notching or a scarlike line or depression
1)

2.

Incomplete cleft lilp

a. b. c.

IV. Classification (Contd)

B.

Location of the cleft: Unilateral versus bilateral

1.
2.

Unilateral cleft lip Bilateral cleft lip

a. May have complete or incomplete cleft on both sides,or a combination b. More likely to be complete clefts and are often wide c. The premaxillary segment may include tooth buds. d. In bilateral complete clefts, the prolabium lacks muscle tissue, and therefore lacks philtral columns.

C.

Alveolar segments
1. 2.

Narrow versus wide cleft Collapse versus no collapse

V.
A.

Syndromes associated with cleft lip and palate

Van der Woudes syndrome

Autosomal dominant, with variable penetrance. 2. Associated with CLP or CP (40%-50% penetrance). 3. Associated with lip pits (accessory salivary glands, 70%-80% penetrance). 4. May also have absent second molar, syndactyly, abnormal genitalia, and popliteal pterygia.
1.

V. Syndromes associated with cleft lip and palate (Contd)

B.

Waardenburgs syndrome
A group of anomalies arising from abnormal development and migration of neural crest cells. 2. Features may include cleft lip, cleft plate.
1.

C. D.

Down syndrome (trysomy 21) Trisome 13

V. Syndromes associated with cleft lip and palate (Contd)

E.

Sticklers syndrome
A group of anomalies caused by connective tissue dysplasia. 2. Typical features: Cleft palate, progressive joint degeneration, and various ocular abnormalities that may lead to blindness. 3. Autosomal dominant inheritance. 4. Other anomalies: Cardiac, sensorineural, and learning disorders or mental retardation.
1.

V. Syndromes associated with cleft lip and palate (Contd)

F.

Pierre Robin sequence (Note: A sequence is a group of anomalies that result from a single disrupted event)
1.

2.
3.

Micrognathia or retrognathia prevents normal descent of the tongue. The tongue then interferes with fusionof the palatal shelves. As a result, typical features include micrognathia or retrognathia, glossoptosis (tongue falls back into the pharynx, causing airway obstruction), and a U-shaped cleft palate. May be a part of multiple different syndromes or may be an isolated finding. Treatment.
Prone positioning to help move the tongue out of the aierway, the most conservative approach. Supplemental oxygen. Tongue-lip adhesion. Mandibular distraction osteogeneisi Intubation/tracheostomy

a. b. c. d. e.

V. Syndromes associated with cleft lip and palate (Contd)

Patients may show catch-up mandibular growth, depending on their syndromic association. 5. Palysomnogram: Necessary to evaluate for desaturations as well as apneic events
4.

V. Syndromes associated with cleft lip and palate (Contd)

G.

Velocardiofacial syndrome
1.

2.

Autosomal dominant inheritance: Fluorescent in situ hybridization (FISH) may show an abnormality in chromosome 22. Characteristic feature include the following.
Cleft palate. Congenital heart disease. Broad nasal dorsum and elongated face. Narrow, down-slanting palpebral fissures. Velopharyngeal insufficiency is common, even with a submucous cleft palate. The carotid arteries may be displaced medially, placing them at high risk of injury during pharyngeal flap surgery or dynamic sphincter pharyngoplasty. Always palpate the posterior pharynx prior to making an incision; consider obtaining a preoperative angiogram.

a. b.

c. d. e.
f.

V. Syndromes associated with cleft lip and palate (Contd)

H.

Median cleft lip

1.
2.

3.

4. 5.

Rare A different entity from the typical cleft lip; more accureately considered a median craniofacial cleft (Tessier type zero). Associated with a group of syndromes (median cerebral facial dysgenesis) that involve more severe deformities of midline CNS and facial structures. Further workup is needed, including a formal CNS evaluation. May be associated with holoprosencephaly, pituitary problems, and a limited lifespan.

VI. Staging of intervention

A.

Initial evaluation
1. 2. 3. 4.

Reassure the parents and family that they are not to blame. Explain the stages and operations that should be expected throughout the childs lifetime Evaluate for associated anomalies. Consultations
Genetics, for evaluation and possible counseling Social works Feeding/nutrition
1) 2)

a. b. c.

d.

Otolaryngology: Children with cleft lip and palate have a high incidence of eustachian tube dysfunction, and therefore otitis media, requiring close follow-up.
1)
2)

The child may need special nipples or bottles (e.g., cross-cut nipple) Monitor for appropriate weight gain

The child may need myringotomy tubes. If unreated, repeat otitis may affect hearing and speech development.

VI. Staging of intervention (Contd)

B. Wide clefts (> 1 cm)
1.

Goal : Bring the segments closer together to facilitate a tension-free repair.

a. b.

Has not been shown to chanbe skeletal development in the anteroposterior direction. Does not seem to prevent future crossbite. Steri-Strip tapes applied across both segments of the lip. Requires reliable parents who can reapply the tape and keep it on at all times

2.

Passive: Preoperative taping

a. b.

VI. Staging of intervention (Contd)

3.

Passive: Lip adhesion operation

a.
b. c.

Suturing the edges of the cleft together is performed under anesthesia. The definitive lip repair is performed once the segments have moved closer together. Variable success.
An orthodontic appliance that must be placed onto the palatal segments under anesthesia. Parents turn a screw daily, which slowly brings the palatal segments into better alignment. Removed at the time of definitive lip repair.

4.

Active: Latham-type device

a. b. c.

VI. Staging of intervention (Contd)

C.

Repair
1.
a. b.

Timing (controversia)

2.

Rule of tens: For increased anesthetic safety, an infant should

a. b. c.

Repair at 3 months is generally accepted. Some argue for earlier repair in order to produce better scars.

3. 4.

Cleft palate repair and secondary alveolar frafting May also choose to address the cleft, nasal deformity at time of lip repair.

Be 10 weeks old Weigh 10 pounds. Have a hemoglobin level of at least 10 mg/dL.

VII. Intraoperative considerations

A.

Landmarks
1.

Tattooed with methylene blue, using a hypodermic needle or a quill pen.

a. b.

c.
d.

e.

Alar bases. Columella. Philtral columns. D. Peak of Cupids bow midline on the medial segment. Measure the anticipated distance for the new Cupids bow (approximately 3-4 mm). Peak of Cupids bow on the lateral segment.

2.

Account for distortion from the uncountered pull of the orbicularis on the medial segment. The philtral columns are usually slightly C-shaped.

VII. Intraoperative considerations (Contd)

Mark lines for expected repair type. C. Only after marks are completed, infiltrate tissue with local anesthetic to avoid distortion of anatomy and measurements. D. Goals of repair
B.
1. 2. 3.

4.
5.

Reconstitute Cupids bow Minimize scarring Produce a slight pout of the tubercle. Produce functional continuity of the muscles Recreate symmetry.

VIII. Types or repair

A.

Straight-line repair
Historically, the first cleft lip repairs relied on freshening the edges of the cleft and suturing them together. These have been largely replace by various Z-plasty-base techniques. 2. Rose-Thompson repair
1.
a.

b.

Modified straight-line repair that can be used for minor clefts with lip length nearly equal on both sides of cleft (e.g. forme fruste) Fusiform excision with straight-line closure.

VIII. Types or repair (Contd)

B.

Quadrangular flap
1.
2. 3. 4. 5.

Proposed by LeMesurier and Hagedorn. Cupids bow is derived from the lateral lip. 90-degree Z-plasty. Violates Cupids bow and the philtral dimple. May also have problem with a long lip.

VIII. Types or repair (Contd)

C.

Triangular flap
1.

Proposed by Tennison and Randall

a. b. c. d. e.

The Z-plasty is place at the vermilion border. Produces an natural appearing Cupids bow May be used for clefts of all widths. Violates Cupids bow and the philtral dimple. Has a tendency to produce a long lip.

2.

Skoog repair
a.
b.

Consists of two Z-plasties Violates Cupids bow and the philtral dimple.

VIII. Types or repair (Contd)

D.

Rotation advancement
1.

Popularized by Millard

a. Likely the most commonly used repair. Often described as the cut-as-yougo technique. b. The medial lip is rotated downward to fill the cleft defet. c. A small pennant-shaped C-flap can either be rotated to create the nasal sill or used to lengthen the columella. d. Does not violate Cupids bow or the philtral dimple____ e. Difficult for wider clefts. f. Common fitfall is inadequate flaprotation leading to nothing and inadequate vertical lip length.
1) 2)

2.

Poople repair
a. b.

Repeat advancement or a small Z-plasty at the vermilion border can be performed. Better results are obtained if adequate rotation is permormed at the time of the original operation.

Preserves the integrity of the aesthetic unit at the columellar-labial junction. Allows lengthening of the lip woithout extending the advancement flap up on the ala or encroaching on horizontal lip length.

VIII. Types or repair (Contd)

E.

Bilateral cleft lip repair

1.
2. 3.

The premaxillary segment is often a greater problem than in a unilateral cleft lip. Consider taping, lip adhesion, or presurgical orthodontics Most common techniques

a. b. c.

d.

Dissect the prolabium to maintain a central skin flap to resemble the philtrum. Deepithelialize the remainder of the prolabium Use the prolabial vermilion to create a labial sulcus, not for the final lip the lateral lip segments, not from the prolabium. Columellar lengthening may be performed at the time of lip repair or as a secondary procedure.

IX. Postoperative care

A.

Orders
Arm restraints (no-nos) for 3 weeks to prevent disruption of repair. 2. Specialized nipple/bottle to decrease sucking effort when bottle-feeding.
1.

B. C.

Leave Steri-Strips in place over the incision for reinforcement. Follow up in 1 week for suture removal if nonabsorbable skin sutures were used.

Pearls
Preoperative 1. Practice lip markings and cuts on foam first 2. Do not forget to assess for an adequate bony platform and the need for orthogmatic surgery when assessing cleft nasal deformities.

Pearls (Contd)
Intraoperative 1. Mark several times, cut once. 2. Beaver scalpel blades are helpful. 3. Line up the white roll first, placing a stitch above and below the white roll, then reapproximate the wet dry border. 4. Bilateral cleft: Do not use the vermilion of the premaxillary segment in the final vermilion. It tends to look like an abnormal, dry patch postoperatively.

Pearls (Contd)
Postoperative 1. Instruct the parents to hold off feeding prior to the clinic appointment. In the clinic, the baby will stay quet during feeding usually just long enough for suture removal. 2. Keep a Steri-Srip tape in place for 1 to 2 weeks for support.